Wegener's granulomatosis affects people of both genders and all ages, though it is very rare in children.
Other parts of the body affected by Wegener's granulomatosis may include:
•Skin - symptoms may include purpura (a raised, red-purple rash), gangrene, or Raynaud's phenomenon, in which fingers, toes, hands, or feet turn white when exposed to cold
•Nerves - numbness, tingling, pain, or weakness in the arms, legs, hands, or feet
•Joints - arthritis
•Eyes - inflammation in the white of the eye (sclera), behind the eye, or in the eye. Complications may include proptosis (the protrusion of one eye) or masses pressing on the optic nerve and causing blindness
Wegener's is associated with an antibody (a protein in the blood) called anti-neutrophil cytoplasmic antibody (ANCA). Blood tests measure the presence of ANCA. Certain diagnostic testing, such as for "casts," protein, or blood in the urine or ANCAs in the blood may help distinguish Wegener's from other types of vasculitis.
The confirmation of Wegener's, however, relies on biopsy of an involved organ. Although a trachea biopsy is less invasive than that of the lung or kidney, it may not produce enough tissue for a definitive diagnosis.
Prior to the 1970s, Wegener's was almost always fatal. Now it is treated with high doses of prednisone in combination with a drug to suppress the immune system and an antibiotic. About half of Wegener's patients experience flares of the disease after an initial remission, so it is important to keep careful watch over symptoms and side effects of medications.